End-stage lung disease patients suffering from systemic sclerosis should not be denied access to lung transplants based on limited survival rate concerns. Those are the conclusions of a new study conducted by Columbia University College of Physicians and Surgeons in New York City and recently presented at the American College of Rheumatology Annual Meeting in Boston. The research revealed that despite extra-pulmonary factors, patients should be able to receive transplantations.
The research team was able to demonstrate that adults suffering from systemic sclerosis, also known as scleroderma, have a survival rate that is one year longer if submitted to lung transplant compared to patients suffering from interstitial lung disease (ILD), and comparable survival rates with pulmonary arterial hypertension (PAH) lung transplant patients. Systemic sclerosis is an autoimmune rheumatic disease that causes thickening and tightening of the skin, as well as inflammation and scarring of other organs, which can lead to malfunction of the lungs, kidneys, heart or intestinal system.
The research was based on a retrospective cohort study, including 3,763 adults who had been submitted to either single or double lung transplantation in the United States between May 4, 2005 and September 14, 2012. The main purpose was to assess the viability of the procedure on patients suffering from the rare disease that strikes between 75,000 and 100,000 Americans, since Hospitals have been reluctant to approve lung transplantation in systemic sclerosis patients over concern that extra-pulmonary involvement factors might affect patients’ short- and long-term survival.
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“Although many transplant centers are hesitant to offer lung transplantation to patients with SSc due to concern about extra-pulmonary involvement that might affect survival, there are very few data to support the impression that survival is worse in SSc patients compared to non-SSc patients,” explained the lead author of the study, Elana J. Bernstein, MD, MSc of Columbia University College of Physicians and Surgeons.
“In fact, previous studies — albeit limited to small case series and small cohort studies — suggest that adults with SSc have similar mortality rates to adults with ILD and PAH following lung transplantation. We actually hoped to confirm the findings of these prior small studies, thereby perhaps providing an impetus for transplant programs to begin to change their practices and offer lung transplantation to greater numbers of carefully selected patients with SSc who are otherwise suitable candidates for lung transplantation,” she added.
To conduct the study, the researchers used data from the United Network for Organ Sharing about patients older than 18 years who were diagnosed with SSc, ILD or PAH, had received transplants at one of the centers, and performed at least one such procedure for SSc. From the 3,763 adults transplanted patients, 229 had SSc, 201 had PAH and, 3,333 had ILD. Patients who had been submitted to a heart-lung transplant, who received a lung from a living donor, or who had missing data on survival time were excluded from the study.
A multivariable-adjusted analysis revealed that SSc was associated with a 48-percent relative increase in the one-year mortality rate when compared to patients diagnosed with ILD. There was no association between SSc and a relative increase in the one-year mortality rate in comparison to PAH, for which lung transplants are widely indicated. Therefore, the researchers recommended the approval of lung transplantation for SSc patients as a treatment option, since they believe that other variables in addition to SSc diagnosis influences risk and survival rates.
“We found that adults with SSc undergoing lung transplantation in the United States during the lung allocation score era had a 48 percent relative increase in the risk of death at one year compared to those with ILD,” Bernstein said. “In contrast, the risk of death at one year among those with SSc was comparable to those with PAH, a widely accepted indication for lung transplantation.”
“In additional analyses among one-year survivors, we did not detect an increased risk of three-year mortality among those with SSc compared to those with ILD, and found that adults with SSc actually had a lower risk of death at three years than those with PAH. Future work should aim to prospectively study adults with SSc as they are evaluated for lung transplantation in order to identify potentially modifiable risk factors that can improve transplant outcomes in this population,” she explained.
The study was partially supported by the Health Resources and Services Administration, and Bernstein’s work was also supported by a New York-Presbyterian Hospital Translational Research Grant.